Mucosal
melanoma of the head and neck is a very rare and aggressive
malignancy with a very poor prognosis. The nasal cavity, paranasal sinuses, and oral cavity are the most common locations. One-, 3- and 5-year survival rates between 2000 and 2007 were 63%, 30% and 20%, respectively. Cigarette smoking seems to be a risk factor even though the evidence for this is very low. Clinical signs and symptoms are usually nonspecific. While surgery is considered the mainstay of treatment for most mucosal
melanomas of the head and neck region,
radiotherapy has a role in local control of the disease after surgery. Many new treatment options in the last years, in particular targeted
therapies (i.e. inhibitors of c-KIT, NRAS/
MEK or BRAF) and
immunotherapies (anti CTLA-4 and anti PD-1/PD-L1
antibodies), have changed the history of cutaneous
melanoma. Despite the different biology, mucosal
melanoma is currently treated in the same way as cutaneous
melanoma; however, patients with mucosal
melanoma were excluded from the majority of recent clinical trials. Recent molecular findings offer new hope for the development of more effective systemic
therapy.